Imaging in Prune Belly Syndrome and Other Syndromes Affecting the Urogenital Tract

Abstract

The absence of the abdominal musculature, urinary tract dilatation, and bilateral undescended testis is known as prune belly syndrome (PBS) (Eagle and Barrett 1950; Greskovich and Nyberg 1988; Williams 1982). The classical syndrome is also known as triad syndrome, Eagle-Barrett syndrome, or abdominal muscular deficiency syndrome. There is a broad spectrum of malformations with severe dilatation of the urinary tract as a consequence of aplasia of the musculature. The pathogenetic mechanism is different from that of dilatation as a consequence of supra- or infravesical obstruction. Some patients with prune belly syndrome have a real obstruction, such as urethral aplasia with oligohydramnios syndrome. The prognosis of the malformations depends upon the degree of renal dysplasia (Rohrmann and Duckett 2011). There is no consensus as to the pathogenesis of this complex abnormality, although most investigators consider prune belly syndrome a distinct entity.